Caso Clínico/Case Report

Acta obstet Ginecol Port 2010;4(3):157-160
Caso Clínico/Case Report
Útero didelfos, hemivagina cega e agenesia renal ipsilateral,
a propósito de um caso clínico
Didelphic uterus and obstructed hemivagina with ipsilateral
renal agenesis: a case report
Rita Abreu*, Marcela Guerra**, Mário Oliveira***, Fernando Mota****
Unidade de Saúde Local do Alto Minho; Hospital Infante D. Pedro EPE, Aveiro;
Hospitais da Universidade de Coimbra
Abstract
Mullerian anomalies comprise a broad range of anomalies. The clinical syndrome of a double uterus,
double cervix with obstruction of the vagina and ipsilateral renal agenesis is rare and a high index
of suspicion is necessary to diagnose this disorder. The authors report a case of a 13-year-old girl
with progressive abdominal pain during menses seven months after menarche. Magnetic resonance
showed a didelphic uterus, right hemihaematometrocolpos and suggested the presence of two vaginas. The patient underwent transvaginal excision of the septum with prompt clinical improvement.
Keywords: Mullerian anomalies, uterus didelphys, Obstructed hemivagina, Renal agenesis, herlyn-Werner-Wunderlich syndrome
iNtRoDuCtioN
Uterus
didelphys with obstructed hemivagina is
an obstructive Mullerian anomaly caused by lateral
nonfusion of the Mullerian ducts with an asymmetric obstruction1. The true incidence of this anomaly
is unknown, but has been reported between 0.1%
and 3.8 %2. This condition is almost always associated with renal agenesis ipsilateral to the obstruction,
which could be attributable to an embryologic arrest
at 8 weeks of gestation that simultaneously affects the
Mullerian and the metanephric ducts1.
It was irst recognized in 1992 and is sometimes
referred to as herly-Werner-Wunderlich (hWW)
syndrome3. This entity is usually diagnosed at puberty, shortly after menarche. Recurrent and progressive pelvic pain is the main clinical complaint.
The clinical presentation may comprise not only a
dilated hemivagina, but also a dilated uterine cavity and a fallopian tube, as well as bleeding into the
peritoneal space as a consequence of blood stasis
and retrograde menstruation in the obstructed system3. Occasionally, patients present with fever, peritonitis, purulent vaginal discharge and leukocytosis,
leading to a misdiagnosis of Pelvic inlammatory
disease (PID)2. Endometriosis can result from blood
relux into the abdominal cavity and may have dire
consequences3. A right sided prevalence of the ob-
*Assistente hospitalar da unidade de Saúde Local do Alto Minho
**Interna de Pediatria do hospital Infante D. Pedro, EPE, Aveiro
***Assistente Graduado de Ginecologia/Obstetrícia, hospital Infante D. Pedro, EPE, Aveiro
****Professor Auxiliar com Agregação e Assistente hospitalar Graduado de Ginecologia/Obstetrícia dos huC
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Abreu R, Guerra M, oliveira M, Mota F
structed system has been described3. An early and
accurate diagnosis of this entity is important, so that
the resection of the obstructing vaginal septum can
provide relief of pain and prevent further complications.
The authors describe a case of a didelphic uterus
and obstructed hemivagina with ipsilateral renal
agenesis whose initial symptom was vulvar discomfort erroneously diagnosed as vulvovaginitis.
Fig.3 T2-W axial MRI image showing dilated right hemivagina
Fig.1 T2-W coronal MRI image showing a didelphic uterus with two
hemicavities
CASE REPoRt
A 13-year-old girl presented to our Emergency Department with a 1-month history of perineal pain,
dysuria, vulvar discomfort and vaginal discharge.
She had been treated with antimycotics, with no improvement, by her General Practitioner. She experienced menarche 7 months before admission, her
menstruation cycles were regular but she complained
of progressive abdominal pain during menses. A gyFig.2 T2-W sagittal MRI axial MRI image showing a right hemihaematometrocolpos
158
Fig.4 Bulge of the anterior vaginal wall from an obstructed right
hemivagina
Acta obstet Ginecol Port 2010;4(3):157-160
Fig.5 Intraoperative photograph showing the drainage of both the
hematocolpos and hematometra
pos, (Fig.2) suggesting the presence of two vaginas
(Fig.3). In the examination carried out under general
anaesthesia, the bulging of the vagina was identiied
as a right transverse vaginal septum (Fig.4) and the
left portion of the vagina had its own cervix and was
free from obstruction. A transvaginal excision of the
septum was performed, about 300 cc of haematic
luid were evacuated (Fig.5) and the right sided cervix was exposed, followed by marsupialization of the
vaginal septum (Fig.6). The postoperative course and
the long-term follow-up were uneventful.
DiSCuSSioN
Fig.6 Intraoperative photograph showing postoperative result after
excision and marsupialization of the vaginal septum
naecologic examination failed to reveal any anomalies of her external genitalia and a uni-digital vaginal
palpation revealed a cystic mass bulging through the
anterior vagina wall with 7x4 cm. An abdominopelvic ultrasound examination disclosed the absence of
the right kidney and a dilated right hemiuterus, and
a magnetic resonance image (MRI) showed a didelphic uterus (Fig.1) and a right hemihaematometrocol-
uterus didelphys with obstructed hemivagina is a
rare entity and a high index of suspicion is required
to identify this uterine anomaly. In a retrospective case review (n=8) the mean age of diagnosis
was 14.5 years with menarche at the mean age of
12.5 years. All the patients had a history of regular
menses with cyclic pelvic pain and the mean time
to diagnosis was 37.8 weeks2. In the present case,
the correct identiication of this situation was one
month delayed due to a misdiagnosis of a mycotic
vulvovaginitis. Perineal pain or vaginal discharge
are not often described as initial symptoms. In this
case, digital pelvic examination provided useful information and raised the suspicion of an obstructive
mullerian anomaly.
Prior to any surgical intervention, imaging studies should be obtained. The evidence of haematocolpos on an ultrasound, appearing as a luid collection
with low-level echoes, permits an easier detection of
the uterine anomaly (didelphic/bicornuate bicollis
uterus)3. It can also conirm the absence of one kidney, which in the presence of an obstructed genital
tract should promptly lead to the correct diagnosis2.
MRI is considered to be more sensitive for imaging
soft tissue compared with Computerised Tomography (CT), however it is unable to accurately assess
the presence of endometriosis, pelvic infection or
adhesions that can affect future fertility2. A few case
series conirm that laparoscopy should be considered the gold standard for the complete evaluation
of congenital anomalies of the female reproductive
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Abreu R, Guerra M, oliveira M, Mota F
tract2. In our patient, the ultrasound conirmed the
presence of two uterine cavities and the absence of
the right kidney and the MRI helped characterizing the
uterine anomaly.
Currently, the preferred surgical approach for patients
with hWW syndrome is the full excision and marsupialization of the vaginal septum. Errors in the surgical management can occur when the diagnosis is not suspected
and a laparotomy resection of the intrabdominal mass is
attempted4. As an alternative to the conventional treatment, a hysteroscopic resection of the vaginal septum
under transabdominal ultrasound guidance can be performed. hysteroscopy considerably enlarges the surgical view which facilitates the preservation of the hymen
integrity and the correction of small hematocolpos1. In
the current case, a classical surgical approach was preferred in order to rapidly relief the perineal pain caused
by a tense right hematocolpos.
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The present case demonstrates that despite the rarity of this entity it was possible to ascertain the correct diagnosis and, therefore, prevent complications
and consequently preserve fertility.
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